Monday, April 24, 2017

Week 192: Keeping up

Now that we've had the G-tube for just over a year, it pretty much runs in the background. For a long time, as we were all getting used to it, every night had the potential for G-tube-related drama, but now we forget that there can even be such a thing. Of course, as soon as you set something on the back burner, it finds a way to remind you of its existence. Case in point being Thursday night, when Lemon was doing naked back-flips off of the couch after his bath, as one does, and somehow caught the edge of the button on some part of the couch and pulled the button right out of his belly. You would think that ripping something out of your belly to reveal a hole that shouldn't be there would be upsetting, but no. Lemon was way more upset about the fact that he had to stop doing back-flips and hold still so that I could put his new tube in than the fact that he'd ripped the old one out. 
On Saturday, while so many of you were out marching for science, I decided to forgo the marching and instead attend our clinic's annual family education day. One of the great highlights was getting to see some of my fellow CF mamas, whom I see way to rarely. In fact, while we were there we were trying to figure out how many times we'd actually seen each other in person after knowing each other for two years. It may be as many as 4 times. And, there was that one time where we went out to lunch together. Once in two years. We're aiming for another lunch in June some time--it's good to set ambitious goals!








Another great experience of the family day was the chance to meet an adult with CF. Thanks to all the advances in CF care (yay science!), more than half of CF patients are now over 18, but the adult CF community and the community of parents of kids with CF seem to be very separate. It was great to get to meet someone in his 30's with CF who is living life to the fullest--career, family, and a passion for running and cycling (he's a faster marathoner than I am), and to be reminded that this kind of future is out there for Lemon too as long as we do everything we can now to give him the best starting point for it.


One really interesting thing I learned from this guy is that in his opinion, manual chest PT is better than the vest. I'd actually heard and read that elsewhere before, but I hadn't really thought about it to deeply. It will be interesting to see as Lemon grows and becomes more able to express what he perceives about his care which therapies he finds work best for him. In the more immediate sense, though, it got me thinking about our travel plans for this summer. We have been trying to figure out how to fly with the vest, since it is too big to fit into the overhead bins of most of the airplanes that fly from our local airport. 

Talking to this adult with CF and hearing his opinion about manual PT made me think--I have to take my hands with me on this trip no matter what. Could we just leave the vest behind? After relying on it pretty much exclusively for almost 2 years, that seems like a daunting prospect. But, on the other hand, it would simplify things so much--imagine if we had to get through security and onto the plane with "only" two children under the age of 4, 1 car seat, 1 giant bag of medicine and food, and all the other assorted accessories, but we didn't have to bring the too-big roll-aboard and then try to negotiate with the airline people to find a spot for it on the plane? 







I have a trip for work coming up in 10 days (two nights with no kids for the first time in 3 years!), but once I'm back I think Lemon and I will start practicing our manual PT technique to see if this is a realistic possibility for us. We'll have to learn some new approaches, since the last time I did manual PT with him he was small enough to do it on my lap--not so anymore!




Monday, April 17, 2017

Week 191: Eat it up

Spring in Wisconsin really is an event. One moment, everything is sort of wet and gray, and the next moment, color bursts forth everywhere, and scantily clad Badgers emerge from their lairs to enjoy the sun. The air has so much pollen in it that it almost has a texture. Thankfully I feel like now, on our third spring here, we finally have a handle on Lemon's seasonal allergies. We're doing Zyrtec twice a day (up from our usual once), Flonase once a day, and running the air purifier whenever he's asleep. So far, so good.











One thing that I noticed this week is that after months of eating basically nothing, Lemon has actually started eating some food. Nothing that you would get excited about if you didn't know his history, but you do, so you will be excited too--on Saturday night at dinner he ate 3 pieces of bow-tie pasta. That is probably more pasta than he has eaten in the last year combined. And, on Easter morning, he ate two Reese's peanut butter eggs. I really do wonder if it's seasonal--maybe he's hungrier because he's outside running around like a madman all day? Or just feeling especially chipper with the nice weather? Whatever it is, I'll take it. Mainly, I'm just hoping that we can capitalize on this extra little bit of willingness when we start feeding therapy next month. If he's somehow in a more receptive mood towards food at the moment, and we do therapy at the same time, maybe we'll really get some traction. A mom can hope, right?





I'm certainly feeling a little more chipper, if for no other reason than that Lime has done me the favor of staying in bed until after 5 a.m. for a week straight--taking his favorite truck to bed with him seems to help. I realize intellectually that the difference between 5:05 and 4:45 is only 20 minutes, but my brain seems to be pretty firmly hard-wired that any a.m. time that begins with a "4" is night, whereas "5" is early but definitely morning. So, 5:05 seems survivable. Lime has also come up with a new game that we can play together at that hour that he finds totally hilarious. It goes like this: Lime points out the (completely dark, pitch black) window in our living room and says "Plane!" and I say, "Really? Do you see a plane?" and he says "Nope!" And then he cracks up. Then we pause for a second. Then he points out the window and says "Moon!" and I say, "Really? Do you see the moon?" And he says "Nope!" And then he cracks up. Then we pause for a second. Then he points out the window and says "Truck!" and before you know it, it's 5:45 and time to get the rest of the family mobilized.

Speaking of before you know it, our annual Great Strides fundraising walk is just a few weeks away. Many of you have already made incredibly generous donations (thank you!!!!). Those of you who haven't done so yet can join the illustrious company of those who have by clicking right here, right now.  And, once you've done that, you can enjoy this video of Lemon, who, having mastered street luge, has moved on to "lawn luge..."




Monday, April 10, 2017

week 190: Sticking with the plan

I'm not going to lie, I'm tired tonight. I ran a half marathon on Saturday, which went really well--I knocked 7 minutes of my time from September. I'm continuing to love the small town races in Wisconsin--the "crowds" (tens of people, I tell you!) are awesome, and the races are gorgeous. Long, beautiful country roads through farmland. But, racing takes its toll. My legs feel great but it definitely took a lot of energy to put together those miles. That, and for the past couple of weeks, Lime has been waking up at 4-4:30 a.m. every day, for no obvious reason. It's pitch dark and dead quiet. He's not hungry. He's still tired. Teeth? Internal clock malfunction? Who knows, I just hope it stops soon. We did a lot of fun stuff this week--proof in the photos, not the words!











Monday, April 3, 2017

Week 189: In bloom

Spring has definitely sprung in Wisconsin, even if the temperature is still be quite chilly. The forsythia has its first tentative yellow blossoms, the garlic and rhubarb in our back yard have sprouted, and my new hobby of leafing through seed catalogs and dreaming is in full effect.

With these blossoms comes our old friend pollen. We know that Lemon has seasonal allergies. We deployed the bedroom air purifier, complete with brand-new HEPA filter. We went up from one daily dose of Zyrtec to two. We added Flonase back to our daily routine. And, I think, by and large, our approach is keeping things in check so far. Lemon still has some coughing and sneezing, but not too much. Of course, my subconscious refuses to update its operating parameters with information about the new season, so every time I hear Lemon cough in his sleep, I bolt awake, with my subconscious saying "Red alert! Cough detected!" Then, it takes a moment or two for my conscious mind to over-ride the distress signal with a "Hush, it's just allergies. And it's 1 a.m."

We continue to make incremental progress towards the new things I'm working to access for Lemon. After several phone calls with 4 different offices, I now have a range of estimates for how much it will cost us to take Lemon for a consult in Milwaukee. Of course, they can't tell you exactly which of the 5 numbers they quoted will be the right one (and actually it's 10 numbers because there's one bill from the hospital for the use of its space, and a second bill from the organization that employs the doctors for the use of the doctor's mind). But, we have a sense of what the minimum and maximum charges are and are comfortable with moving forward. So, I'm hoping to find time to reconnect with the social worker there and actually schedule something for June, once Lemon is done with school for the year.

We're also incrementally closer to getting started with feeding therapy. Our insurance agreed to cover at least the first few sessions, which is great, so I need to fill out a few more forms (there are ALWAYS more forms) and then we can schedule his two-hour evaluation. We're supposed to bring a selection of several foods that he will eat, and several foods that he's likely to reject. Suffice to say, the latter category will not be an issue. He did totally surprise me by eating several pieces of fresh mango at dinner tonight, so that's something.

This week featured some exciting news from the CF research world: Vertex announced the results of two clinical trials on its latest drug combination that treats the root cause of CF. You can read the press release here. One trial tested the drug combination in patients with two copies of the common F508del mutation, and the other tested the same combination in patients with one copy of F508del and one "residual function" mutation. A residual function mutation is one in which the CFTR protein (that is missing or defective in patients with CF) isn't totally missing/defective. Patients in both studies saw a significant improvement in lung function compared to patients who took the placebo, and patients with two copies of the F508del mutation also had a very significant reduction in pulmonary exacerbations. This is very exciting, even though Lemon's second mutation isn't traditionally classified as a "residual function" mutation. It does mean that we are one step closer to a drug combination that will directly help him--take a look here to see all the exciting things that the CF Foundation has coming down the line.

As you all know by now, research costs money. We're in the midst of our annual Great Strides fund raiser now, to help provide the CF Foundation with the resources it needs to continue this amazing work. To those of you who have already donated to our campaign, thank you, thank you, and thank you. For those of you who haven't donated yet, please visit our fundraising page. With the announcement of these latest clinical trials, we're so tantalizingly close to the medicine that Lemon desperately needs--please help us get there!



Monday, March 27, 2017

Week 188: For the record

This week, we began our adventure in eating therapy. On the recommendation of our dietitian, I contacted a private clinic here in Madison that does feeding therapy and set up an introductory consultation and visit to the clinic. The day of the appointment arrived, and I was extremely encouraged by the following piece of dialog that occurred as I attempted to convince the star of the show that we should leave the house:
Lemon: Where are we going? 

Mama Bear: We are going to a special clinic to talk to someone about eating, to see if we can find more things that you like to eat. 

Lemon: Will there be toys there?

Mama Bear: Yes, there will probably be some toys in the waiting room that you can play with. And then we will go to talk to someone about eating.

Lemon: And I will tell them, "NO!"

On that note, we drove over to the clinic where, thankfully, there were a bunch of toys that Lemon could look at. We met with someone on the staff there who did a quick interview to assess our situation. I gave her a summary of Lemon's eating habits, such as they are, and then she asked me to list some foods that he will eat. I listed red pepper, donut, pepperoni, ice cream, apple, cheese, and Pirate's Booty. She reviewed the list and said, "Well, he doesn't seem to have a problem with textures at least." So, that seemed positive. After touring the facility, we went home to complete a three-day eating diary and a bunch of other forms. I will now share with you, for the record, the entirety of what Lemon has consumed by mouth in the last 72 hours:




















Saturday: 2/3 slice white toast with Nutella; 1 gum ball (this came on the top of a rather magnificent-looking Easter-themed donut where the gum ball represented an egg, and I cannot tell you how delighted Lemon was when I explained to him that gum was a food that you chewed and then spit into a napkin instead of swallowing, he could scarcely believe his luck); 1/4 slice American cheese, 1/8 slice pepperoni, 6 1-inch cubes cantaloupe.

Sunday: 2 cubes of watermelon, 2 pieces of Pirate's Booty, 1/3 juice box (at his friend's birthday party); 3 tablespoons chocolate ice cream

Monday: 4 Teddy Grahams, 1 oz milk; 1 oz milk

That's it. Maybe 400 calories in 3 days if you round up. Kind of a long way from the 1500-2000 calories a day that he needs. So, this marks our starting point, and we'll see where we go from here. At least we have a lot of room for improvement?


Papa Bear joked that just for comparison I should keep a 3-day diary for Lime, but I can't simultaneously do that and prevent him from snatching a stick of butter off the counter and taking a big bite out of it, so I'll have to pass. 

Thanks so much to everyone who has donated to our annual Great Strides campaign so far. If you haven't yet, don't worry, there's still time--just head right on over to our team page and make your contribution. And if you're local and want to actually walk the walk with us, go ahead and sign up as a team member, we'd love to have you!




Monday, March 20, 2017

Week 187: Live long and prosper

Had you asked me 3 months ago whether we would make it from one regularly scheduled clinic visit to the next without a sick visit or a hospitalization in between (given that the months in question were January, February, and March), I would have laughed at you. But, thanks to the power of Cayston, we did it. Our appointment went quite well, another minor miracle. Somehow, without really telling me about it, Lemon managed to pack on a pound and a half between when I weighed him at home last and when we got to the clinic. So, although if you average out from December until now, he isn't quite meeting his weight gain goal, he isn't doing half badly, either. We talked with our dietitian, and decided not to go up on the tube feed amount for now--we'd like to keep Lemon a little hungry, just on the off-chance that he decides to eat any solid food ever. On that note, I've got him scheduled for an evaluation at a feeding clinic later this week, so more on that soon I imagine.

After the clinic visit part of the appointment was over, we stayed on to help out with a research study. I would say participate, but it wasn't exactly that. As many of you know, a key measure of disease severity in CF is pulmonary function, which can be measured by two tests. Our center is going to participate in a new study that requires the use of these tests in kids aged 3-6, so they need their operators to prove that they can get accurate results in kids that young. When we got called to participate, I was a little skeptical, since Lemon is not exactly at the most cooperative stage of life, but I'm vehemently pro-research so I figured we should try to do whatever we could to help.

So, after the clinic appointment, we went to the pulmonary function lab to see what Lemon would do. He actually did much better than I expected at the first test, aided by a computer screen that displayed a birthday cake with candles that went out when he blew in the tube correctly. I think we got at least one trial on that was good enough to help certify our clinic staff. The other test was not as successful--that one required having a mask form an air-tight seal on his face for several consecutive minutes. He wasn't afraid of having a mask over his face, but he had to sit really, really still in order for the mask to maintain its seal. And sitting really, really still is something he is really, really not good at right now. So, for that test, we weren't able to get a run that was good enough to count towards certifying the operator. Honestly, watching both tests made me wonder how accurate they can be in kids as young as Lemon--it seems like we really need some other ways to measure lung function in kids this age.

In other news (literally--this story has been picked up a lot of places including the New York Times and the Washington Post), a study came out last week that found that patients with CF in Canada have a median survival that is 10 years longer than in the US. Both countries had similar survival up until around 2005, when the length of survival in Canada started to pull away from the US. There are a number of factors that may have contributed to this difference. The Canadians figured out the importance of a high-fat, high-calorie diet for CF patients earlier on (in the 1970's) and it took some time for that finding to be broadly applied throughout the US. So, adults with CF in Canada may be reflecting a benefit of that early childhood nutrition that the US patients missed. A higher percentage of Canadian CF patients get life-extending lung transplants than is seen in the US (although even in Canada, the percentage of CF patients who get a lung transplant is pretty low, just over 10%). So, what is behind this big difference? A key clue is that patients in the US who have private health insurance live just as long as Canadian ones (who all are covered under Canada's single-payer healthcare system). In other words, access to healthcare matters tremendously, and the importance of healthcare access is made starkly and unequivocally clear by a challenging medical condition like CF.

This study makes another important point, though, which is that everyone benefits from the study of CF and other rare diseases. Rare diseases like CF are the ones that have detailed registries to enable research like the study we just talked about--research that drew on almost 3 decades of meticulous record-keeping and follow-up. Rare diseases are at the cutting edge of personalized medicine and gene therapy, leading the way for strategies to tackle more common ailments like cancer and diabetes. Rare diseases are at the forefront of health economics, testing how the tremendous cost of these personalized medications can be borne in the face of finite resources.

Of course, to me, research on rare diseases is more than a societal good, and more than a scientific interest. To me, it is a very personal passion. The progress that has extend the lifespan of CF patients from 5 years in 1950 to 40.6 years (in the US) or 50.9 years (in Canada) today is astonishing, but obviously there is more work to be done. And the registries, the research on genetics, the development and testing of drugs, the design of gene therapies? That stuff is expensive. In this day and age, when federal support for medical research may be wavering, research supported by private foundations like the Cystic Fibrosis Foundation is more important than ever. So, please click here and donating to our Great Strides team to sustain these life-saving efforts. It's incredibly important to our family, and--in ways that we can't necessarily anticipate yet--it will be important to yours.







Monday, March 13, 2017

Week 186: The year of the tube

Hard though it may be to believe, it has now been a full year since Lemon had his G-tube placed. A year since I spent 6 nights in a hospital with a toddler and an infant (no, I don't have any recollection of how that worked). A year of figuring out how to use this new tool that we'd been given to wield in our fight with CF. It's a fitting time to reflect--just last night, I spent an hour on the phone with another mama who is standing where I stood a year ago, trying to figure out if taking this big step would be the right decision. For us, I think there can be little doubt that it was, but the road between the day of the surgery and today has not been an easy one.

A year ago, Lemon weighed 25.4 lb and was in the 6th percentile for weight. Today, he's 36 lb, and in the 80th percentile. Before, he was in the 30th percentile for height, and now he's in the 70th. Before, we couldn't get enough vitamins and fat into him to get him to be even at the lowest end of the "normal" range on a number of nutritional tests. Now, we've had to cut back his daily multivitamin dose by half so that he doesn't exceed the normal range. The numbers speak for themselves.

But, the tube is a tool, not a magic wand, and it's taken us some time figure out how to use it. We had to figure out what a "normal" g-tube site looks like, what an infection looked like, what granulation tissue looked like, and what do do about each of those things. We had to figure out how much formula Lemon could tolerate, and how fast, and how many hours had to elapse between the end of the tube feed and the start of his morning therapy. We had to learn how to do daytime bolus feeds, and figure out how to incorporate them into our daily routine. We had to learn how to swap out the G-tube itself when the old button was worn out (and what worn out even means in a G-tube button). We had to let Lemon learn the wet and messy consequences of disconnecting the tube during the night.

I think the biggest lesson we had to learn was about how to use the tube when he was sick. I had the idea going into this that the tube would allow us to keep him fully nourished while he was ill. For him, at least, that's simply not the case. Tube + illness = vomiting. It took us a few times to absorb that lesson, but I think we've mostly got it now. What the tube does mean for us is that we don't lose quite as much ground when he's sick as we used to, and whatever ground we do lose we can make up very quickly. It also means that we can keep him hydrated and bypass ye olde gag reflex when giving bad-tasting medicines, both of which are really invaluable benefits.

I'd really hoped that, with the tube, Lemon would learn how to eat. I thought that if we could just take the pressure off of him, and let him eat whatever he wanted, not just rich, high calorie foods, that he would find things he liked and enjoy eating them. Not so. If anything, I feel like the security of the tube has let him be as weird about food as he likes, because some part of his reptile brain knows that he's getting the calories over night, so there's no incentive to eat actual food during the day. From the beginning, I'd imagined it would be a few years at least before we'd be ready to wean him off the tube, but now I really wonder if even that estimate was optimistic. Now that we're used to the tube, though, I'm in no rush to get him off of it. I suppose some day, he'll be ready. Or not: there are plenty of adults with CF who have tubes and do overnight feeds to keep up with their caloric needs. But, once he turns 18, I'm not getting up at 3 a.m. anymore to to turn off the pump!

I almost never plan blog posts in advance, but with the anniversary of Lemon's surgery this week, I'd been carrying around a sketch of this week's post in my mind all day. Of course, the one time I actually have a plan, an exciting research paper come out the same day. But, it would be too long a post to do both things at once (and a girl's gotta sleep sometime) so I'll talk about the research paper next week. It ties into a lot of themes from my day job and current events so I"m excited to write about it and give it the space that it needs. So, do your homework, read the abstract for the study in the Annals of Internal Medicine, and come back next week!